Pediatric Cranial Vault Pathology.

A wide variety of diagnoses can be approached with a common framework for diagnosis, extirpation, and reconstruction of pediatric cranial vault pathologies. Durability of reconstruction is critical for the range of pediatric patients from infancy to adolescence. Rigid reconstruction, preferably with autologous tissue when possible, promotes brain protection and satisfactory aesthetic outcome. Careful planning can allow for immediate definitive reconstruction of defects without need for further surgical intervention.

A Road Map to Creating a High-Quality Clinical Database in Plastic Surgery.

BACKGROUND: Detailed in-house databases are a staple of surgical research and a crucial source of data for many studies from which clinical guidelines are built. Despite the importance of generating a clear and thorough developmental design, the literature on database creation and management is limited. In this article, the authors present their stepwise single-institution process of developing a clinical facial fracture database. METHODS: The authors outline the process of development of a large single-institution clinical pediatric facial fracture database. The authors highlight critical steps from conception, regulatory approval, data safety/integrity, human resource allocation, data collection, quality assurance, and error remediation. The authors recorded patient characteristics, comorbidities, details of the sustained fracture, associated injuries, hospitalization information, treatments, outcomes, and follow-up information on Research Electronic Data Capture. Protocols were created to ensure data quality assurance and control. Error identification analysis was subsequently performed on the database to evaluate the completeness and accuracy of the data. RESULTS: A total of 4451 records from 3334 patients between 2006 and 2021 were identified and evaluated to generate a clinical database. Overall, there were 259 incorrect entries of 120,177 total entries, yielding a 99.8% completion rate and a 0.216% error rate. CONCLUSIONS: The quality of clinical research is intrinsically linked to the quality and accuracy of the data collection. Close attention must be paid to quality control at every stage of a database setup. More studies outlining the process of database design are needed to promote transparent, accurate, and replicable research practices.

Pediatric Nasoorbitoethmoid Fractures: A Single Institution's 15-Year Experience.

BACKGROUND: Nasoorbitoethmoid (NOE) fractures impact growth of the craniofacial skeleton in children, which may necessitate differentiated management from adult injuries. This study describes characteristics, management, and outcomes of NOE fractures in children seen at a single institution. METHODS: A retrospective review of patients under 18 years who presented to our institution from 2006 to 2021 with facial fractures was conducted; patients with NOE fractures were included. Data collected included demographics, mechanism of injury, fracture type, management, and outcomes. RESULTS: Fifty-eight patients met inclusion criteria; 77.6% presented with Manson-Marcowitz Type I fractures, 17.2% with Type II, and 5.2% with Type III. The most common cause of injury was motor vehicle accidents (MVAs, 39.7%) and sports (31%). Glasgow Coma Scale and injury mechanism were not predictive of injury severity in the pediatric population ( P =0.353, P =0.493). Orbital fractures were the most common associated fractures (n=55, 94.8%); parietal bone fractures were more likely in Type III fractures ( P =0.047). LeFort III fractures were more likely in type II fractures ( P =0.011). Soft tissue and neurological injuries were the most common associated injuries regardless of NOE fracture type (81% and 58.6%, respectively). There was no significant difference in type of operative management or in the rates of adverse outcomes between types of NOE fractures. CONCLUSIONS: These findings suggest that pediatric NOE fractures, although rare, present differently from adult NOE fractures and that revisiting predictive heuristics and treatment strategies is warranted in this population.

Frontofacial Features of Unilateral Lambdoid Craniosynostosis: A Multicenter Assessment.

Unilateral lambdoid craniosynostosis is differentiated from deformational plagiocephaly primarily by assessing the cranium from posterior and bird's-eye views. Findings include posterior displacement of the ipsilateral ear, ipsilateral occipitomastoid bossing, ipsilateral occipitoparietal flattening, contralateral parietal bossing, and contralateral frontal bossing. Diagnosis based off facial morphology may be an easier approach because the face is less obstructed by hair and head-coverings, and can easily be assessed when supine. However, frontofacial characteristics of unilateral lambdoid craniosynostosis are not well described. Methods: A retrospective cohort review of patients with isolated, unilateral lambdoid craniosynostosis from the Children's Hospital of Pittsburgh and the Children's Hospital of Philadelphia was performed. Preoperative frontal and profile photographs were reviewed for salient characteristics. Results: Nineteen patients met inclusion criteria. Eleven patients had left lambdoid craniosynostosis, and eight had right lambdoid craniosynostosis. All patients were nonsyndromic. Patients demonstrated contralateral parietal bossing and greater visibility of the ipsilateral ear. Contralateral frontal bossing was mild. The orbits were tall and turricephaly was present in varying severity. Facial scoliosis as a C-shaped deformity was present in varying severity. The nasal root and chin pointed to the contralateral side. Conclusions: The combination of greater visibility of the ipsilateral ear, contralateral parietal bossing, and C-shaped convex ipsilateral facial scoliosis are hallmark frontofacial features of unilateral lambdoid craniosynostosis. Although the ipsilateral ear is more posterior, the greater visibility may be attributed to lateral displacement from the mastoid bulge. Evaluation of long-term postoperative results is needed to assess if this pathognomonic facial morphology is corrected following posterior vault reconstruction.

Characterization of the Saddle Nose Deformity Following Endoscopic Endonasal Skull Base Surgery.

Objective The endoscopic endonasal approach (EEA) is commonly employed in skull base surgery for neoplasm resection. While nasal deformity following EEA is described, this study aimed to perform a detailed qualitative and quantitative assessment of the associated saddle nose deformity (SND) in particular. Setting/Participants This is a retrospective review of 20 adult patients with SND after EEA for resection of skull base tumors over a 5-year period at the University of Pittsburgh Medical Center. Main Outcome Measures Fifteen measurements related to SND were obtained on pre- and postoperative imaging. Statistical analyses were performed to evaluate differences between pre- and postoperative anatomies. Results The most common EEA was transsellar. Reconstruction techniques included nine free mucosal grafts alone, eight vascularized nasoseptal flaps (NSFs), one combined free mucosal graft/abdominal fat graft, and one combined NSF/fascia lata graft. Imaging analysis showed a trend toward loss of mean nasal height, nasal tip projection, and nasolabial angle postoperatively. Subgroup analysis showed that patients with NSF reconstruction had a significantly decreased nasal tip projection (1.2 mm, p = 0.039) and increased alar base width (1.2 mm, p = 0.046) postoperatively. Patients without functional pituitary microadenomas demonstrated significantly increased nasofrontal angle and decreased nasal tip projection on postoperative imaging, in contrast to those with functional adenomas who had no measurable significant changes. Conclusion Clinically evident SND does not always lead to significant radiographic changes. This analysis suggests that patients who undergo surgery for indications other than functional pituitary microadenomas or who receive NSF reconstruction develop more marked SND on standard imaging tests.

Quantifying the Severity of Metopic Craniosynostosis Using Unsupervised Machine Learning.

BACKGROUND: Quantifying the severity of head shape deformity and establishing a threshold for operative intervention remains challenging in patients with metopic craniosynostosis (MCS). This study combines three-dimensional skull shape analysis with an unsupervised machine-learning algorithm to generate a quantitative shape severity score (cranial morphology deviation) and provide an operative threshold score. METHODS: Head computed tomography scans from subjects with MCS and normal controls (5 to 15 months of age) were used for objective three-dimensional shape analysis using ShapeWorks software and in a survey for craniofacial surgeons to rate head-shape deformity and report whether they would offer surgical correction based on head shape alone. An unsupervised machine-learning algorithm was developed to quantify the degree of shape abnormality of MCS skulls compared to controls. RESULTS: One hundred twenty-four computed tomography scans were used to develop the model; 50 (24% MCS, 76% controls) were rated by 36 craniofacial surgeons, with an average of 20.8 ratings per skull. The interrater reliability was high (intraclass correlation coefficient, 0.988). The algorithm performed accurately and correlates closely with the surgeons assigned severity ratings (Spearman correlation coefficient, r = 0.817). The median cranial morphology deviation for affected skulls was 155.0 (interquartile range, 136.4 to 194.6; maximum, 231.3). Skulls with ratings of 150.2 or higher were very likely to be offered surgery by the experts in this study. CONCLUSIONS: This study describes a novel metric to quantify the head shape deformity associated with MCS and contextualizes the results using clinical assessments of head shapes by craniofacial experts. This metric may be useful in supporting clinical decision making around operative intervention and in describing outcomes and comparing patient population across centers.

Delayed Presentation of Unicoronal Craniosynostosis-Masked by Ipsilateral Posterior Deformational Plagiocephaly.

Despite a greater awareness of the differential diagnosis of head shape abnormalities among pediatricians, the effect of deformational forces on calvarial morphology can complicate the diagnosis of craniosynostosis. In this report, we describe 2 patients diagnosed with unicoronal craniosynostosis (UCS) in a delayed fashion due to the presence of concomitant posterior deformational plagiocephaly (PDP). In both cases, the severity of each patients' PDP obscured changes typically associated with UCS. This unique presentation underscores the importance of having a high index of suspicion for possible premature suture fusion despite the presence of concomitant PDP.

Mandibular Measurements at the 20-Week Anatomy Ultrasound as a Prenatal Diagnostic Predictor of Pierre Robin Sequence.

BACKGROUND: Pierre Robin Sequence (PRS) is characterized by micrognathia, glossoptosis, and upper airway obstruction. Early recognition and appropriate perinatal management is crucial for optimizing outcomes. This study aimed to evaluate 20-week fetal ultrasounds to determine if specific mandibular measurements could predict PRS diagnosis and disease severity. METHODS: A retrospective case-control study of 48 patients with PRS and gender-matched controls was performed. Medical records were reviewed for respiratory and surgical interventions. Three parameters to assess micrognathia were measured on mid-sagittal profile ultrasound images: frontal nasal-mental angle (FNMA), facial-maxillary angle (FMA), and alveolar overjet. Student's t-test and univariate logistic regression was performed. P ≤ 0.05 was considered statistically significant. RESULTS: Patients with PRS demonstrated a significantly smaller mean FNMA compared to the control group, 129.3 ± 8.6° and 137.4 ± 3.2°, respectively (p < 0.0001), as well as significantly smaller mean FMA, 63.2 ± 9.2° and 74.8 ± 6.1°, respectively (p < 0.0001). The PRS group also demonstrated significantly larger mean alveolar overjet compared to the control group, 3.9 ± 1.4 mm and 2.1 ± 0.9 mm, respectively (p < 0.0001). The odds of respiratory intervention increased among cases when FMA was <68°. Additionally, there was a significant difference in median overjet between patients with PRS who did and did not require respiratory intervention. CONCLUSIONS: Mandibular features on the 20-week ultrasound can be measured to predict diagnosis and severity of PRS. This is an important first step to prepare for potential respiratory intervention at delivery to minimize perinatal hypoxia. Alveolar overjet, previously not described in prenatal ultrasound literature, is measurable and has utility in prenatal screening for PRS, as do FMA and FNMA.

Tissue Augmenting Palatoplasty for the Treatment of Velopharyngeal Insufficiency.

PURPOSE: Persistent velopharyngeal insufficiency (VPI) following primary palatoplasty remains a difficult problem to treat. This study evaluates speech outcomes following revision palatoplasty with tissue augmentation using buccal myomucosal flaps (BMF) as an alternative to pharyngoplasty for patients with VPI. METHODS: A retrospective single-center review of revision palatoplasty with tissue augmentation at a tertiary pediatric hospital Cleft-Craniofacial Center between January 2017 and March 2021 was conducted. Patients with a history of previous palatoplasty, a diagnosis of persistent or recurrent VPI, and comprehensive pre- and postoperative speech evaluations who underwent revision palatoplasty with BMF were included. RESULTS: Twenty patients met inclusion criteria (35% female, 20% syndromic). Mean age at the time of revision palatoplasty with BMF was 9.7 years. Preoperatively, all patients had stigmatizing speech and received the recommendation for speech surgery; the mean Pittsburgh Weighted Speech Score (PWSS) was 14.3 ± 4.9. The mean postoperative PWSS at the most recent assessment was 4.2 ± 2.3, representing a statistically significant improvement from preoperative scores (P < .001). Mean follow-up time was 8.9 months. Following revision palatoplasty with BMF, only one patient has received the recommendation for further speech surgery. No complications were noted. CONCLUSION: In patients with VPI following primary palatoplasty, revision palatoplasty with tissue augmentation offers an alternative to pharyngoplasty. This approach preserves dynamic velopharyngeal function, improves speech outcomes, and should be considered an option when treating patients with post-primary palatoplasty VPI.

The Impact of the COVID-19 Pandemic on Cleft Care.

BACKGROUND: The COVID-19 pandemic had multiple effects on the provision of health care, including the suspension of elective and nonessential surgeries. The objective of this study was to determine the early effect of the COVID-19 pandemic on the surgical care of patients with cleft lip and/or palate at a high-volume cleft center. METHODS: A retrospective comparative cohort study of patients with cleft lip and/or palate undergoing lip adhesion, cleft lip and nose repair, and palatoplasty before and during the pandemic was conducted. There were 50 patients in the prepandemic cohort and 53 in the pandemic cohort. RESULTS: Mean age at lip adhesion was 3.1 ± 1.1 months prepandemic (n = 8) and 3.5 ± 2.5 months in the pandemic cohort (n = 8) (P = 0.75). One lip adhesion was delayed by 1.6 months. Mean age at cleft lip and nose repair was 6.6 ± 1.9 months prepandemic (n = 23) and 8.0 ± 2.1 months in the pandemic cohort (n = 23) (P = 0.03). Six pandemic cleft lip and nose repairs were delayed; the mean delay was 2.6 ± 1.8 months. The mean age at palatoplasty was 13.9 ± 2.2 months prepandemic (n = 26) and 14.1 ± 2.9 months in the pandemic cohort (n = 26) (P = 0.79). Seven pandemic palatoplasties were delayed; the mean delay was 3.3 ± 1.4 months. CONCLUSIONS: The COVID-19 pandemic caused delays at each stage of repair for cleft lip and/or palate-related procedures; however, only cleft lip and nose repair were significantly affected. This study emphasizes the importance of remaining vigilant regarding the care of this vulnerable population during this challenging time.

Posterior Cranial Vault Distraction Osteogenesis Utilizing a Posterior-Superior Distraction Vector in the Treatment of Mercedes Benz Pattern Craniosynostosis.

ABSTRACT: Bilateral lambdoid and sagittal synostosis, or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in frontal bossing, a tapered posterior fossa, and an anteriorly displaced cranial vertex. Its ideal surgical correction must result in posterior expansion, skull elongation, and caudal repositioning of the vertex. We present a craniometric analysis of skull changes with posterior-superior distraction and introduce a novel craniometric measure: vertex position. In this study, a retrospective review was performed to analyze outcomes of posterior cranial vault distraction osteogenesis (PVDO) using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0). Four patients underwent PVDO at mean age 10.61 ±â€Š3.16 months utilizing a posterior-superior distraction vector. Linear distraction distance averaged 30.30 ±â€Š0.90 mm with a mean consolidation period of 3.98 ±â€Š0.72 months. Mean corrected change in intra-cranial volume was 236.30 ±â€Š3.71 mL, at an average rate of 7.81 ±â€Š2.00 mL/mm of distraction. Increases in anterior cranial height (7.83 ±â€Š2.51 mm), middle cranial height (8.43 ±â€Š4.21 mm), posterior cranial height (13.15 ±â€Š7.45 mm), and posterior cranial fossa height (21.99 ±â€Š8.55 mm) were observed. Cranial vertex demonstrated a mean posterior movement of 0.18 ±â€Š0.13. PVDO utilizing a posterior-superior distraction vector for management of nonsyndromic bilateral lambdoid and sagittal synostosis effectively increases intracranial volume and height and provides an esthetic outcome with posterior movement of the cranial vertex.

Decompressive Cranial Vault Remodeling in Osteosclerotic Robinow Syndrome.

We present a novel application of endocranial burr contouring for cranial vault expansion as a surgical adjunct during decompressive craniectomy in patients with cranial osteosclerosis. A 16-year-old female with osteosclerotic Robinow syndrome complicated by slit ventricle syndrome presented with refractory intracranial hypertension following external ventricular drain placement. Symptoms included severe headaches and altered mental status. Given the severe intracranial volume restriction secondary to massive calvarial thickening (2.5 cm), the patient was taken to the operating room for urgent surgical decompression. After frontal and parietal craniectomy, burr and osteotome contouring were used to remove two-thirds of the endocranial calvarial bone flap thickness resulting in a 9% cranial vault expansion while preserving an overall normal head size. There were no immediate postoperative complications. At over 3 years postoperatively, the patient had reduced headaches, maintained adequate shunt function, and has not required further vault reconstruction.

Surgical Management of Hand Deformity in Epidermolysis Bullosa: Initial Experience and Technique.

Epidermolysis bullosa describes a rare group of genetic mucocutaneous disorders characterized by excessive epithelial fragility resulting in mechanically induced blistering and abnormal wound healing.1,2 Its prevalence and incidence are 8.2 and 19.6 per 1,000,000 live births, respectively.2 Gene therapy, protein replacement, and cell therapy strategies have been investigated, but there is currently no cure.2.

An Intraoperative Salvage After Transection of the Greater Palatine Artery During Cleft Palate Repair: A Case for Buccal Fat and Buccal Myomucosal Flaps.

Recently, several adjunctive procedures have gained traction to aid cleft surgeons in repairing especially challenging palatal clefts. Buccal fat flaps and buccal myomucosal flaps have demonstrated particular utility in reinforcing thin palatal flaps or tissue deficits. Although their use has not been widely accepted, they may be particularly helpful in the setting of significant scarring or vascular compromise. Here the authors describe the case of an intraoperative salvage using bilateral buccal fat flaps and a right buccal myomucosal flap after transection of the right Greater Palatine artery (GPA) during palatoplasty on a 14-month old female with Pierre Robin Sequence and a wide Veau II cleft palate. For this operative salvage, bilateral buccal fat flaps were used to reinforce the hard-soft palate junction and a 4 cm × 2 cm flap of the right-sided buccal mucosa and buccinator muscle was inset along the majority of the right-sided soft and posterior hard palate. At 2 years follow-up, the patient had no significant complications and was doing well with healthy-appearing palatal tissue and age-appropriate speech.